Diagnostic efficacy of ERCP in cholestatic infants and neonates – a retrospective study on a large series

Keil, Radan; Šnajdauf, J; Rygl, Michal; Pýcha, K; Kotalova, Radana; Drábek, Jir̆ı́; Šťovíček, Jan; Procke, Michal

doi:10.1055/s-0029-1215372

Cited by 67 publications

(60 citation statements)

References 10 publications

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“…When biliary atresia is suspected, ERCP may serve as a reliable, accurate and safe additional beneficial diagnostic tool in unclear cases of children with non-pigmented stool (37, 41, 42). Here, ERCP clarified the diagnosis in 73% of the cases where biliary atresia was suspected (16/22 infants).…”

Section: Discussionmentioning

confidence: 99%

Diversity of Disorders Causing Neonatal Cholestasis â€“ The Experience of a Tertiary Pediatric Center in Germany

et al. 2014

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Background and Objective: Rapidly establishing the cause of neonatal cholestasis is an urgent matter. The aim of this study was to report on the prevalence and mortality of the diverse disorders causing neonatal cholestasis in an academic center in Germany.Methods: Clinical chemistry and cause of disease were retrospectively analyzed in 82 infants (male n = 42, 51%) that had presented with neonatal cholestasis to a tertiary medical center from January 2009 to April 2013.Results: Altogether, 19 disorders causing neonatal cholestasis were identified. Biliary atresia was the most common diagnosis (41%), followed by idiopathic cases (13%), progressive familial intrahepatic cholestasis (PFIC, 10%), cholestasis in preterm infants (10%), α1AT deficiency, Alagille syndrome, portocaval shunts, mitochondriopathy, biliary sludge (all 2%), and others. Infants with biliary atresia were diagnosed with a mean age of 62 days, they underwent Kasai portoenterostomy ~66 days after birth. The majority of these children (~70%) received surgery within 10 weeks of age and 27% before 60 days. The 2-year survival with their native liver after Kasai procedure was 12%. The time span between Kasai surgery and liver transplantation was 176 ± 73 days. Six children (7%), of whom three patients had a syndromic and one a non-syndromic biliary atresia, died prior to liver transplantation. The pre- and post-transplant mortality rate for children with biliary atresia was ~12 and ~17%, respectively.Conclusion: Neonatal cholestasis is a severe threat associated with a high risk of complications in infancy and it therefore requires urgent investigation in order to initiate life saving therapy. Although in the last 20 years new causes such as the PFICs have been identified and newer diagnostic tools have been introduced into the clinical routine biliary atresia still represents the major cause.

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Section: Discussionmentioning

confidence: 99%

Diversity of Disorders Causing Neonatal Cholestasis â€“ The Experience of a Tertiary Pediatric Center in Germany

et al. 2014

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“…The efficacy of ERCP in the diagnosis and treatment of traumatic pancreatic duct injury has been well documented in adults [47][48][49]. ERCP in infants is a safe and reliable procedure that can detect biliary tract abnormalities when performed by an experienced endoscopist [50,51].…”

Section: Discussionmentioning

confidence: 99%

What is the role of endoscopic retrograde cholangiopancreatography in assessing traumatic rupture of the pancreatic in children?

Keil

Drábek

Lochmannová

et al. 2015

Scandinavian Journal of Gastroenterology

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“…Histology is characterized by inflammatory cell infiltrates around bile ducts, portal tract fibrosis, accumulation of bile typically presenting as bile plugs, and bile duct proliferation. ERCP may serve as a reliable and safe additional diagnostic tool (10, 26, 27) exhibiting a sensitivity of up to 86–92% and a specificity of 73–94% (10, 28). Hepatobiliary sequence scintigraphy is associated with a high sensitivity (83–100%) but lacks specificity in many cases (33–80%), limiting its usefulness to discriminate between BA and other non-surgical conditions (29).…”

Section: Introductionmentioning

confidence: 99%

“…According to Todani, five anatomic variants have been described, but most infants show dilatation of the common bile duct (Todani type 1, 50–80% of biliary cysts) (41). Caroli’s disease is associated with multifocal, segmental, and saccular or fusiform dilatations of the medium and large intrahepatic bile ducts without affecting the common bile duct (28). These malformations may be limited to one liver lobe, most commonly the left one.…”

Section: Introductionmentioning

confidence: 99%

“…This condition may also include renal abnormalities, e.g., autosomal recessive polycystic kidney disease (ARPKD), juvenile nephronophtisis, Joubert syndrome, and others (42). The diagnosis is established by ultrasound, ERCP, and/or MRCP (28). Administration of ursodeoxycholic acid (UDCA) decreases bile stasis and prevents the formation of intrahepatic cholelithiasis.…”

Section: Introductionmentioning

confidence: 99%

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Neonatal Cholestasis â€“ Differential Diagnoses, Current Diagnostic Procedures, and Treatment

et al. 2015

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Cholestatic jaundice in early infancy is a complex diagnostic problem. Misdiagnosis of cholestasis as physiologic jaundice delays the identification of severe liver diseases. In the majority of infants, prolonged physiologic jaundice represent benign cases of breast milk jaundice, but few among them are masked and caused by neonatal cholestasis (NC) that requires a prompt diagnosis and treatment. Therefore, a prolonged neonatal jaundice, longer than 2 weeks after birth, must always be investigated because an early diagnosis is essential for appropriate management. To rapidly identify the cases with cholestatic jaundice, the conjugated bilirubin needs to be determined in any infant presenting with prolonged jaundice at 14 days of age with or without depigmented stool. Once NC is confirmed, a systematic approach is the key to reliably achieve the diagnosis in order to promptly initiate the specific, and in many cases, life-saving therapy. This strategy is most important to promptly identify and treat infants with biliary atresia, the most common cause of NC, as this requires a hepatoportoenterostomy as soon as possible. Here, we provide a detailed work-up approach including initial treatment recommendations and a clinically oriented overview of possible differential diagnoses in order to facilitate the early recognition and a timely diagnosis of cholestasis. This approach warrants a broad spectrum of diagnostic procedures and investigations including new methods that are described in this review.

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Diagnostic efficacy of ERCP in cholestatic infants and neonates – a retrospective study on a large series

Cited by 67 publications

References 10 publications

Diversity of Disorders Causing Neonatal Cholestasis â€“ The Experience of a Tertiary Pediatric Center in Germany

Diversity of Disorders Causing Neonatal Cholestasis â€“ The Experience of a Tertiary Pediatric Center in Germany

What is the role of endoscopic retrograde cholangiopancreatography in assessing traumatic rupture of the pancreatic in children?

Neonatal Cholestasis â€“ Differential Diagnoses, Current Diagnostic Procedures, and Treatment

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